Case Report Acute Marjolin s Ulcer in a Postauricular Scar after ...downloads.hindawi.com/journals/criot/2016/2046954.pdf · Case Report Acute Marjolin s Ulcer in a Postauricular

Case ReportAcute Marjolinrsquos Ulcer in a Postauricular Scarafter Mastoidectomy

Kholoud A Alhysoni Sumaiyah M Bukhari and Mutawakel F Hajjaj

Otolaryngology Department Ohud Hospital Medina Saudi Arabia

Correspondence should be addressed to Kholoud A Alhysoni kholoudalhysonihotmailcom

Received 6 September 2016 Accepted 17 November 2016

Academic Editor Abrao Rapoport

Copyright copy 2016 Kholoud A Alhysoni et al This is an open access article distributed under the Creative Commons AttributionLicense which permits unrestricted use distribution and reproduction in any medium provided the original work is properlycited

Background Marjolinrsquos ulcer is a rare aggressive cutaneous malignancy that arises primarily in burn scars but can occur in othertypes of scars Squamous cell carcinoma is the most common variant and while malignant degeneration usually takes a long timeit can develop acutely Case Report a 30-year-old man who developed Marjolinrsquos ulcer acutely in a right postauricular scar aftermastoidectomy and the incision and drainage of a mastoid abscess To the best of our knowledge this report is the first to describea Marjolinrsquos ulcer in a postauricular surgical scar However it has been reported in others areas in the head and neck ConclusionMarjolinrsquos ulcer is most commonly observed after postburn scars but it may be observed after any type of scars as our patientdeveloped an SCC with a postsurgical scar Early diagnosis is essential and a biopsy should be performed on any nonhealingwound or chronic wound that undergoes a sudden change Tissue samples should be taken from both the centre and the marginsof the wound

1 Introduction

Marjolinrsquos ulcer refers to cancer that most often presentsin an area with a chronic burn wound Marjolinrsquos ulceris also associated with nonhealing wounds venous ulcerslupus vulgaris vaccination scars snake bite scars chronicosteomyelitis fistulae [1] amputation stumps cystostomysites chronic lymphedema chronic pilonidal sinuses [2]pressure ulcers in spina bifida patients [3] ischial bursitis[4] hidradenitis suppurativa [5] posttraumatic scars [6ndash9]surgical scars [10] and scars after coronary artery bypassgrafting [11]

The most commonly affected sites are the lower extrem-ities followed by the head and neck region and the trunk[1 11] Themost commonly involved areas of the head are thescalp [1 6] and face [1 10] in one reported case the nose wasaffected [12] and in another the neck was affected [8]

We report the case of a 30-year-old man who developedMarjolinrsquos ulcer in the right postauricular area only 9 monthsafter the incision and drainage of a right mastoid abscessThere are no other reports of Marjolinrsquos ulcers in this area todate

2 Case Presentation

A 30-year-old Bangladeshi male presented to the emergencyroom with a five-day history of right postauricular swellingthat had gradually increased in size There was associatedfever and purulent discharge from the right ear

The patient had a longstanding history of right eardischarge and decreased hearing in the right ear with notinnitus or vertigo

The patient had no medical illness and was negative forhuman immunodeficiency virus

Examination revealed a right mastoid swelling that wasfluctuant hyperemic tender and warm Needle aspirationrevealed 6 cc of purulent fluid The right external auditorycanal (EAC) and right tympanic membrane perforationemitted purulent discharge The facial nerve was intact onexamination with no palpable lymph nodes

Pure tone audiometry showed right profound mixedhearing loss

Axial computerized tomography of the temporal boneshowed a mastoid abscess with bone destruction (Figure 1)

Hindawi Publishing CorporationCase Reports in OtolaryngologyVolume 2016 Article ID 2046954 5 pageshttpdxdoiorg10115520162046954

2 Case Reports in Otolaryngology

(a) (b)

Figure 1 Axial CT scan (noncontrast) showing the temporal bone (a) Bone window and right ill-defined soft tissue density in the rightmastoid air cells middle and inner ear associated with bony destruction Only the basal turn of the cochlea and part of the vestibuleare visualized (b) Soft tissue window and posterior aspect of the tegmentum tympani appear destroyed thinned and interrupted withsubcutaneous soft tissue swelling adjacent to the EAC and collection at the superior aspect

Magnetic resonance imaging of the brain showedenhanced collection in the subcutaneous tissue and auricularregion posterior and anterosuperior to the EAC that extendedto themastoid cavity and themiddle ear cleftThere was focalarea of dural enhancement observed in the right temporallobe (Figure 2)

A diagnosis of right chronic suppurative otitis mediacomplicated by mastoid abscess was made

Incision and drainage of the right mastoid abscesswith modified radical mastoidectomy were performed andrevealed that a large cholesteatoma sac occupied the mastoidcavity and extended to the middle ear cleft The histopathol-ogy results were consistent with cholesteatoma (Figure 3)

Postoperatively the patient developed right postauricularwound dehiscence The patient was lost to follow-up forseven months and later presented with 4 cm by 5 cm rightpostauricular ulcer with raised edges and a necrotic centre(base) (Figure 4)

Computerized axial tomography showed a right periau-ricular soft tissue mass with an ill-defined border (Figure 5)

Biopsies were taken from the edges and centre of thelesion The biopsy from the edges showed moderately differ-entiated squamous cell carcinoma and those from the centreshowed dysplasia with keratinous material (Figure 6)

As the patient after diagnosis chose to return to his homecountry no definite treatment was given to him

3 Discussion

Marjolinrsquos ulcer is a rare and often aggressive cutaneousmalignancy that develops in previously traumatized orchronically inflamed skin particularly after burns [7]

In the first century Aurelius Cornelius Celsuswas the firstto report the development of a tumour in old burn scars andchronic nonhealing wounds In 1828 Jean Nicholas Marjolina French surgeon described a phenomenon that involvedthe formation of ulcerations within a burn scar and coinedthe term ldquoulcere cancroiderdquo however the description did notsay that the ulcers were malignant [13] In 1838 Dupuytrenobserved that de novo malignancy could arise in chronicwounds he observed this phenomenon in a Belgianmanwhowas treated for a cancer that developed from a scar sustained

from a sulphuric acid burn [14] The name ldquoMarjolinrsquos ulcerrdquowas first used by Da Costa in 1903 when he defined an ulcerarising from burn scars as Marjolinrsquos ulcer [15]

Squamous cell carcinoma is the most common histo-logical type among these wounds followed by basal cellcarcinoma sarcoma and melanoma [2 10 11 16] The male-to-female ratio increases with increasing patient age over 50years [10 11 16]

Various theories have been proposed to explain thepathogenesis of the malignant transformation of thesewounds but none has provided a full explanation Thetoxins theory which proposes that the chronic inflammatoryprocesses that lead to tissue damage produce toxins that maybe carcinogenic was proposed by Treves and Pack [11] Vir-chowrsquos theory of chronic irritation explains that with chronicirritation and repeated tissue injury the epithelium becomesless stable loses contact inhibition and undergoes malignantchange Other proposed theories include epithelial elementimplantation (Ribetrsquos theory) the cocarcinogenic theory(Friedwald and Rose) and the immunologically privilegedsite theory Castillo and Goldsmith suggested that the poorlymphatic flow in scar tissue impairs immunosurveillancemaking it difficult for the body tomount an effective antigen-antibody response to protooncogens or tumours withinscars Hereditary theory and the environmental and geneticinteraction theory seek to explain the evolution of acuteMarjolinrsquos ulcers by suggesting that genetic differences makethe individual more susceptible to environmental insultsresulting in a short latency period

As none of the above theories fully explain the evolutionof Marjolinrsquos ulcer some studies have proposed a multifacto-rial theory consisting of various combinations of the currenttheories [3 17 18]

Latency has been described as the time between theprimary pathology and the confirmation of a pathologicdiagnosis of Marjolinrsquos ulcer The reported latency periodfor the development of malignancy is between 11 and 75years [18] Marjolinrsquos ulcer can be classified as acute orchronic In acute ulcers the malignant degeneration occurswithin 12 months in the more common chronic ulcers thedegeneration occurs after 12 months

Case Reports in Otolaryngology 3

(a) (b)

(c) (d)

Figure 2 MRI of the brain IAC and mastoid with IV contrast Axial and coronal views show (a) MRI T1 axial view before contrast (bc) MRI T1 axial view after contrast and (d) MRI T1 coronal view after contrast Right large loculated peripheral enhancing collection isobserved in the subcutaneous tissue of the auricular region posterior and anterosuperior to the external auditory canal and extending to themastoid air cells and middle ear cavity The cochlea and semicircular canals are not visualized only part of the vestibule is observed and afocal area of dural enhancement is observed in the right temporal lobe

Figure 3 Cholesteatoma

Figure 4 A 4 cm by 5 cm right postauricular ulcer with raised edgesand a necrotic centre

When acute the ulcer is most often basal cell carcinomaand is associated with a more superficial burn scar Howeveracute malignant transformations to SCC do occur [5 10 19]Many cases of acute transformation ranging from weeks [1820] to months [7 14 21] have been reported in the literature

Regarding the age of the patient and the burn scar patientage is inversely proportional to the interval to the formationof cancerThe younger the patient is themore likely he or sheis to have a latency period of less than 1 year older patientsare increasingly likely to have a latency period greater than 1year [10 22 23]

Marjolinrsquos ulcer tends to be more aggressive than othertypes of skin cancer and has a higher rate of regional metas-tases [10] However head and neck lesions are associatedwith better survival as are lesions of the upper extremitiesOther factor associated with better survival include a latencyto malignancy of less than 5 years ulcers caused by burnschronic osteomyelitis a tumour size less than 2 cm and ulcersless than 4mm in thickness [3 11]

Early diagnosis is essential A high index of suspicionshould be considered in the presence of chronic ulcerspersisting longer than 3 months rolled or everted woundmargins foul-smelling discharge and an increase in painulcer size or bleeding [7 9 18 22 24] Biopsy of suspiciouslesions for histopathology remains the gold standard fordiagnosis [24] Many studies have recommended biopsiesof multiple areas such as the centre and margins [19] atappropriate depths [11]

Treatment of Marjolinrsquos ulcer is quite varied To preventwound degeneration into squamous cell carcinoma it isimperative to provide early and definitive wound cover-age after burns and other traumatic injuries Leaving largewounds to heal by secondary intention creates the potentialfor a chronic nonhealing ulcer and the ideal conditions fordevelopment of a Marjolinrsquos ulcer Wide local excision andsubsequent skin grafting appear to be the standard of care


(a) (b)

Figure 5 CT scan of the temporal bone shows that compared to previous images the soft tissue component was increased causing furtherdestruction of the middle and inner ear and a right periauricular soft tissue mass lesion with an ill-defined border

Figure 6 Section shows proliferative squamous cells invading the underlying stroma The cells are hyperchromatic with a high NC ratioand atypical mitosis

for most authors [8] MU is more aggressive than primaryskin tumours therefore nodal assessment and wide surgicalexcision are recommended [1]

4 Conclusions

Marjolinrsquos ulcer is most commonly observed after postburnscars but it may be observed after any type of scars as ourpatient developed an SCC with a postsurgical scar Earlydiagnosis is essential and a biopsy should be performed onany nonhealing wound or chronic wound that undergoes asudden change Tissue samples should be taken fromboth thecentre and the margins of the wound

Additional Points

Summary We presented a case of a 30-year-old man whodeveloped Marjolinrsquos ulcer acutely in a right postauricularscar after mastoidectomy and the incision and drainage of amastoid abscess To the best of our knowledge this reportis the first to describe a Marjolinrsquos ulcer in a postauricularsurgical scar Early diagnosis is essential and a biopsy shouldbe performed on any nonhealing wound or chronic woundthat undergoes a sudden change

Competing Interests

The authors declare no competing interests

Acknowledgments

The authors are grateful to AhmedAlhujaily histopathologistat King Fahad Hospital and Talal Almoghthawey radiologistat Ohud Hospital

References

[1] N Yu X Long J R Lujan-Hernandez et al ldquoMarjolinrsquos ulcera preventable malignancy arising from scarsrdquo World Journal ofSurgical Oncology vol 11 article 313 2013

[2] MGOnesti P Fino P Fioramonti VAmorosi andN ScuderildquoTen years of experience in chronic ulcers and malignanttransformationrdquo International Wound Journal vol 12 no 4 pp447ndash450 2015

[3] P M Nthumba ldquoMarjolinrsquos ulcers theories prognostic factorsand their peculiarities in spina bifida patientsrdquoWorld Journal ofSurgical Oncology vol 8 no 1 article 108 2010

[4] A H Cruickshank E M Mcconnell and D G Miller ldquoMalig-nancy in scars chronic ulcers and sinusesrdquo Journal of ClinicalPathology vol 16 pp 573ndash580 1963

[5] J B Chang T A Kung and P S Cederna ldquoAcute marjolinrsquosulcers a nebulous diagnosisrdquo Annals of Plastic Surgery vol 72no 5 pp 515ndash520 2014

[6] COzekNCelikU Bilkay TAkalinO Erdem andACagdasldquoMarjolinrsquos ulcer of the scalp report of 5 cases and review of theliteraturerdquo Journal of Burn Care amp Rehabilitation vol 22 no 1pp 65ndash69 2001

[7] P L Chalya J B Mabula J M Gilyoma P Rambau N Masaluand S Simbila ldquoEarly Marjolinrsquos ulcer developing in a penile


human bite scar of an adult patient presenting at BugandoMedical Centre Tanzania a case reportrdquo Tanzania Journal ofHealth Research vol 14 no 4 pp 288ndash292 2012

[8] M A Kerr-Valentic K Samimi B H Rohlen J P Agarwal andW B Rockwell ldquoMarjolinrsquos ulcer modern analysis of an ancientproblemrdquo Plastic and Reconstructive Surgery vol 123 no 1 pp184ndash191 2009

[9] M E Asuquo I A Ikpeme G Ebughe and E E BasseyldquoMarjolinrsquos ulcer sequelae of mismanaged chronic cutaneousulcersrdquo Advances in Skin ampWound Care vol 23 no 9 pp 414ndash416 2010

[10] M S Fazeli AH LebaschiMHajirostam andMR KeramatildquoMarjolinrsquos ulcer clinical and pathologic features of 83 cases andreview of literaturerdquo Medical Journal of the Islamic Republic ofIran vol 27 no 4 pp 215ndash224 2013

[11] V Challa V Deshmane and M Ashwatha Reddy ldquoA retro-spective study of Marjolin1015840s ulcer over an eleven year periodrdquoJournal of Cutaneous and Aesthetic Surgery vol 7 no 3 p 1552014

[12] E Copcu and N Culhaci ldquoMarjolinrsquos ulcer on the noserdquo Burnsvol 28 no 7 pp 701ndash704 2002

[13] T Wojewoda W Wysocki and J Mitu ldquoMarjolinrsquos ulcermdashcasereport and literature reviewrdquo Polish Journal of Surgery vol 81no 9 pp 414ndash418 2009

[14] D Thio J H W Clarkson A Misra and S SrivastavaldquoMalignant change after 18 months in a lower limb ulcer acuteMarjolinrsquos revisitedrdquo British Journal of Plastic Surgery vol 56no 8 pp 825ndash828 2003

[15] J Da Costa ldquoIII Carcinomatous changes in an area of chroniculceration or Marjolinrsquos ulcerrdquo Annals of Surgery vol 37 no 4pp 496ndash502 1903

[16] M Bozkurt E Kapi S V Kuvat and S Ozekinci ldquoCurrentconcepts in the management of Marjolinrsquos ulcers outcomesfrom a standardized treatment protocol in 16 casesrdquo Journal ofBurn Care amp Research vol 31 no 5 pp 776ndash780 2010

[17] KOpara and IOtene ldquoMarjolinrsquos ulcers a reviewrdquoTheNigerianHealth Journal vol 11 no 4 pp 107ndash111 2011

[18] B Pekarek S Buck and L Osher ldquoA comprehensive review onMarjolinrsquos ulcers diagnosis and treatmentrdquo The Journal of theAmerican College of CertifiedWound Specialists vol 3 no 3 pp60ndash64 2011

[19] A Mohammadi S Sayed Jafari and M Hosseinzadeh ldquoEarlyMarjolinrsquos ulcer after minimal superficial burnrdquo Iranian Journalof Medical Sciences vol 38 no 1 pp 69ndash70 2013

[20] A N Wooldridge M J Griesser T Scharschmidt and O HansIwenofu ldquoDevelopment of Marjolinrsquos ulcer within one monthof burn injury with synchronous primary lung squamouscell carcinoma in an elderly patient report of a case withallelotypingrdquo Medical Oncology vol 28 no 1 pp S586ndashS5922011

[21] E Celik H Fyndyk and A Uzunismail ldquoEarly arising Mar-jolinrsquos ulcer report of three casesrdquo British Journal of PlasticSurgery vol 58 no 1 pp 122ndash124 2005

[22] U Ochenduszkiewicz R Matkowski B Szynglarewicz andJ Kornafel ldquoMarjolinrsquos ulcer malignant neoplasm arising inscarsrdquo Reports of Practical Oncology and Radiotherapy vol 11no 3 pp 135ndash138 2006

[23] S B Hahn D J Kim and C H Jeon ldquoClinical study ofMarjolinrsquos ulcerrdquo Yonsei Medical Journal vol 31 no 3 pp 234ndash241 1990

[24] S Enoch D R Miller P E Price and K G Harding ldquoEarlydiagnosis is vital in the management of squamous cell carcino-mas associated with chronic non healing ulcers a case seriesand review of the literaturerdquo International Wound Journal vol1 no 3 pp 165ndash175 2004

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Hindawi Publishing Corporationhttpwwwhindawicom Volume 2014


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PPAR Research

The Scientific World JournalHindawi Publishing Corporation httpwwwhindawicom Volume 2014

Immunology ResearchHindawi Publishing Corporationhttpwwwhindawicom Volume 2014

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Gastroenterology Research and Practice


Parkinsonrsquos Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttpwwwhindawicom


(a) (b)

Figure 1 Axial CT scan (noncontrast) showing the temporal bone (a) Bone window and right ill-defined soft tissue density in the rightmastoid air cells middle and inner ear associated with bony destruction Only the basal turn of the cochlea and part of the vestibuleare visualized (b) Soft tissue window and posterior aspect of the tegmentum tympani appear destroyed thinned and interrupted withsubcutaneous soft tissue swelling adjacent to the EAC and collection at the superior aspect

Magnetic resonance imaging of the brain showedenhanced collection in the subcutaneous tissue and auricularregion posterior and anterosuperior to the EAC that extendedto themastoid cavity and themiddle ear cleftThere was focalarea of dural enhancement observed in the right temporallobe (Figure 2)

A diagnosis of right chronic suppurative otitis mediacomplicated by mastoid abscess was made

Incision and drainage of the right mastoid abscesswith modified radical mastoidectomy were performed andrevealed that a large cholesteatoma sac occupied the mastoidcavity and extended to the middle ear cleft The histopathol-ogy results were consistent with cholesteatoma (Figure 3)

Postoperatively the patient developed right postauricularwound dehiscence The patient was lost to follow-up forseven months and later presented with 4 cm by 5 cm rightpostauricular ulcer with raised edges and a necrotic centre(base) (Figure 4)

Computerized axial tomography showed a right periau-ricular soft tissue mass with an ill-defined border (Figure 5)

Biopsies were taken from the edges and centre of thelesion The biopsy from the edges showed moderately differ-entiated squamous cell carcinoma and those from the centreshowed dysplasia with keratinous material (Figure 6)

As the patient after diagnosis chose to return to his homecountry no definite treatment was given to him

3 Discussion

Marjolinrsquos ulcer is a rare and often aggressive cutaneousmalignancy that develops in previously traumatized orchronically inflamed skin particularly after burns [7]

In the first century Aurelius Cornelius Celsuswas the firstto report the development of a tumour in old burn scars andchronic nonhealing wounds In 1828 Jean Nicholas Marjolina French surgeon described a phenomenon that involvedthe formation of ulcerations within a burn scar and coinedthe term ldquoulcere cancroiderdquo however the description did notsay that the ulcers were malignant [13] In 1838 Dupuytrenobserved that de novo malignancy could arise in chronicwounds he observed this phenomenon in a Belgianmanwhowas treated for a cancer that developed from a scar sustained

from a sulphuric acid burn [14] The name ldquoMarjolinrsquos ulcerrdquowas first used by Da Costa in 1903 when he defined an ulcerarising from burn scars as Marjolinrsquos ulcer [15]

Squamous cell carcinoma is the most common histo-logical type among these wounds followed by basal cellcarcinoma sarcoma and melanoma [2 10 11 16] The male-to-female ratio increases with increasing patient age over 50years [10 11 16]

Various theories have been proposed to explain thepathogenesis of the malignant transformation of thesewounds but none has provided a full explanation Thetoxins theory which proposes that the chronic inflammatoryprocesses that lead to tissue damage produce toxins that maybe carcinogenic was proposed by Treves and Pack [11] Vir-chowrsquos theory of chronic irritation explains that with chronicirritation and repeated tissue injury the epithelium becomesless stable loses contact inhibition and undergoes malignantchange Other proposed theories include epithelial elementimplantation (Ribetrsquos theory) the cocarcinogenic theory(Friedwald and Rose) and the immunologically privilegedsite theory Castillo and Goldsmith suggested that the poorlymphatic flow in scar tissue impairs immunosurveillancemaking it difficult for the body tomount an effective antigen-antibody response to protooncogens or tumours withinscars Hereditary theory and the environmental and geneticinteraction theory seek to explain the evolution of acuteMarjolinrsquos ulcers by suggesting that genetic differences makethe individual more susceptible to environmental insultsresulting in a short latency period

As none of the above theories fully explain the evolutionof Marjolinrsquos ulcer some studies have proposed a multifacto-rial theory consisting of various combinations of the currenttheories [3 17 18]

Latency has been described as the time between theprimary pathology and the confirmation of a pathologicdiagnosis of Marjolinrsquos ulcer The reported latency periodfor the development of malignancy is between 11 and 75years [18] Marjolinrsquos ulcer can be classified as acute orchronic In acute ulcers the malignant degeneration occurswithin 12 months in the more common chronic ulcers thedegeneration occurs after 12 months


(a) (b)

(c) (d)










(a) (b)




4 Conclusions


Additional Points


Competing Interests


Acknowledgments


References
































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of

















(a) (b)

(c) (d)










(a) (b)




4 Conclusions


Additional Points


Competing Interests


Acknowledgments


References
































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of

















(a) (b)




4 Conclusions


Additional Points


Competing Interests


Acknowledgments


References
































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of








































of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of





















of






Disease Markers



OncologyJournal of





PPAR Research



Journal of

ObesityJournal of
















Documents

Case Report Acute Marjolin s Ulcer in a Postauricular Scar after ...downloads.hindawi.com/journals/criot/2016/2046954.pdf · Case Report Acute Marjolin s Ulcer in a Postauricular